Institute of Medicine, University of Bergen, 5021 Bergen, Norway.
J Clin Immunol. 2012 Apr;32(2):230-7. doi: 10.1007/s10875-011-9617-4. Epub 2011 Nov 30.
Patients with autoimmune polyendocrine syndrome type I (APS I) or acquired thymoma-associated myasthenia gravis (MG) surprisingly share several common features, including defective expression of the transcription factor AIRE and autoantibodies against type I interferons. Here, we have adapted and validated the radioligand-binding assay we recently developed against (35)S-Met-interferon-ω, for rapid and specific screening for autoantibodies against interferons-α2 and -α8. We then investigated their potential for diagnosis and for predicting clinical manifestations in patients with APS I and different subgroups of MG. Autoantibodies against interferons-ω, -α2, and -α8 occurred more often in patients with APS I (100%) and MG with thymoma (73%) than in late-onset MG (39%) and early-onset MG (5%). These autoantibodies showed preferences for interferon-ω in APS I and for the interferon-αs in MG, hinting at thymic aberrations in both groups. The exact profile of type I interferon antibodies may indicate MG subtype and may hint at thymoma recurrence.
患有自身免疫性多内分泌腺综合征 I 型(APS I)或获得性胸腺瘤相关重症肌无力(MG)的患者令人惊讶地具有一些共同特征,包括转录因子 AIRE 的表达缺陷和针对 I 型干扰素的自身抗体。在这里,我们对最近针对(35)S-Met-干扰素-ω 开发的放射性配体结合测定法进行了适应性和验证,以便快速和特异性筛选针对干扰素-α2 和 -α8 的自身抗体。然后,我们研究了它们在 APS I 患者和不同 MG 亚组中用于诊断和预测临床表现的潜力。针对干扰素-ω、-α2 和 -α8 的自身抗体在 APS I(100%)和胸腺瘤 MG(73%)患者中的发生率高于迟发性 MG(39%)和早发性 MG(5%)患者。这些自身抗体在 APS I 中偏爱干扰素-ω,在 MG 中偏爱干扰素-α,提示两组均存在胸腺异常。I 型干扰素抗体的确切谱可能表明 MG 亚型,并可能提示胸腺瘤复发。